1、北京协和中枢神经系统脱髓北京协和中枢神经系统脱髓鞘疾病鞘疾病Myelin in CNS is formed by the oligodendrocytesChemical composition:proteolipid,myelin basic protein,2-3 cyclic nucleotide phosphohydrolase,myelin-associated glyco-protein,myelin-oligodendrocyte glyco-protein.Intact myelin is required for action potential conductionMyel
2、ined nerve fiber are rich in white matter of cerebral、cerebella、brain stem、spinal cord，optic nerveDemyelinating myelin is broken,axon remains intactDemelinated disorders in CNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMONMOADEMBalosInflammatory demyelinating disorders in c
3、entral nerve systemMultiple Sclerosis(MS)Multiple Sclerosis(MS)1234 Multiple Sclerosis (MS)Multifocal lesionsMultifocal lesionsThe Diagnostic of MS （Poser,1983)Number of AttackEvidence of More Than One LesionClinicalLab.CSF OCB or IgGA.Clinically DefiniteB.Lab-Supported DefiniteC.Clinically Probable
4、D.Lab-Supported ProbableA1 2 2A2 2 1 and 1B1 2 1 or 1 +B2 1 2 +B3 1 1 and 1 +C1 2 1C2 1 2C3 1 1 and 1D1 2 0 0 +Optic nerve atrophy lProgressive mutifocal leukoencephalopathylCentral pontine myelinolysislMarchiafava-Bignami diseaselToxic demyelination:chemical agents,COlIrradiation encephalopathylCerebral vascular diseaseX-linked recessive demyelination of cerebral white matter adrenal insufficiency(unresponsive to ACTH)CT:white-matter dz:occipital regions-frontal lprogression-generalized atrophy MRI:hypointense T1/hyperintense T2,atrophic splenium of corpus callosum
